Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupuserythematosus - A case report

Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto dis ease is a rar e entity, and even more rarely, it is associated with other diseases. In a few cases, the condition is associated with cutaneous lupus erythematosus ( CLE). Histiocytic necrotizing lymphadenitis predominantly affects young wom en, who present with solitary or multiple cervical lymphadenopathy accompan ied by symptoms such as fryer, weight loss, sweating, or, in exceptional ca ses, hepatosplenomegaly. Laboratory ex aminations show normal or nonspecifi c results. The disease is of unknown origin, although a viral origin has be en suggested, with the suspected agents including Epstein-Barr virus, herpe svirus type 6, and cytomegalovirus (CMV). Although the first and most of th e more recent cases have been reported in Oriental patients, the disease ha s a wide geographic distribution. The clinical evolution is favorable, with spontaneous remission in less than 4 months in almost all cases. We presen t a case of a 37-year-old woman from Peru who presented with cervical adeno pathies on two occasions. Biopsy of a lymph node revealed a histopathologic picture compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphaden itis. The adenopathy disappeared in a few months. A year later, she present ed with a maculopapular rash in the nasal and malar regions. The results of the skin biopsy and immunofluorescence examination were compatible with ch ronic CLE. The results of the serology testing for CMV were positive. Treat ment with chloroquine was initiated, with almost complete recovery by 5 mon ths. No manifestations of systemic lupus erythematosus have occurred since. The epidemiologic, clinical, and anatomopathologic aspects as well as the differential diagnosis of this entity are reviewed.